Atrophoderma vermiculatum (AV) is a rare follicular dermatosis presenting with ‘wormeaten’ or ‘honeycomb’ atrophy, usually on the cheeks and sometimes extending to the preauricular areas and forehead. It predominantly affects children and follows a slowly progressive course. The treatment of AV is challenging. We describe a rare case of unilateral AV in a young boy. The scarring in AV is characteristically limited to the face, but our patient had lesions extending to the concha auriculae and the neck. He also had an ipsilateral congenital cataract. Such an association has only been reported once before in the literature.